Dystrophies of the Fundus

Question 1

Retinitis Pigmentosa signs

Answer 1

Optic nerve pallor, Arteriolar attenuation, Bone-spicule pigmentation
chronological order:
arteriolar narrowing mild pigmentary changes
coarse, perivascular bone spicule pigmentation w optic atrophy
increase in density of pigments w. anterior and posterior spread

Question 2

Retinitis Pigmentosa prognosis

Answer 2

Signs and symptoms may worsen over time, may not go blind

Question 3

Best Vitelliform signs in stages

Answer 3

Stage 0: previteliform (normal fundus and subnormal electrooculogram)
Stage 1: Pigment mottling at macula
Stage 2 (vitelliform): round egg yolk macula lesion
Stage 3 (pseudohypopyon): lesion becomes absorbed
Stage 4 (vitlleruptive): 'scrambled egg' VA drops

Question 4

Best Vitelliform prognosis

Answer 4

Good until fifth decade of life, VA declines and patients become legally blind

Question 5

Stargardt Disease signs

Answer 5

Macular Mottling, Oval, snail slime, beaten bronze fovea, surround with yellow white flecks, geography atrophy may be bull’s eye configuration

Question 6

Stargardt Disease prognosis

Answer 6

Once VA is 6/12, VA drops rapidly to 6/60

Question 7

Fundus Flavimaculatus Signs

Answer 7

Bilateral, ill-defined, yellow-white deep retinal flecks

New lesions develop as older ones become softer

Question 8

Fundus Flavimaculatus prognosis

Answer 8

Good until retinal fleck develops at fovea, may develop choroidal neovascularisation which has poor prognosis

Question 9

Managements

Answer 9

Refer patient for genetic counselling, Provide or refer patients with low vision assements or aids, yearly follow up to monitor progress